What is the initial workup and treatment approach for a patient presenting with esophageal spasms?

Esophageal Spasm Workup

Begin with cardiac evaluation to exclude ischemic heart disease, then proceed with barium esophagram, upper endoscopy with biopsies, and high-resolution manometry to establish the diagnosis of esophageal spasm. 1

Initial Evaluation: Exclude Cardiac Disease First

• Cardiac evaluation must be completed before accepting an esophageal diagnosis, given the substantially greater morbidity and mortality of ischemic heart disease compared to esophageal disorders 1
• Esophageal spasms present with squeezing retrosternal chest pain that can mimic cardiac ischemia, making this distinction critical 1
• Only after cardiac causes are ruled out should you proceed with esophageal-specific workup 1

Diagnostic Algorithm for Esophageal Spasm

Step 1: Barium Esophagram (Biphasic Preferred)

• Videofluoroscopy/barium studies have 80-89% sensitivity and 79-91% specificity for diagnosing esophageal motility disorders including diffuse esophageal spasm compared with manometry 2
• Biphasic esophagram (combining double-contrast and single-contrast views) provides superior detection of both mucosal lesions and motility abnormalities 2
• Barium studies may occasionally reveal dysmotility not detected on manometry 2
• This test is complementary to manometry and helps visualize structural abnormalities 3

Step 2: Upper Endoscopy with Biopsies

• Endoscopy is essential to exclude structural causes including tumors, strictures, rings, eosinophilic esophagitis, and infectious esophagitis 2
• Obtain at least 5-6 biopsies even if mucosa appears normal endoscopically to evaluate for eosinophilic esophagitis (≥15 eosinophils per high-power field) 2, 4
• Look for frothy retained secretions and puckered gastroesophageal junction suggesting poor esophageal clearance 2
• Careful retroflexed examination is mandatory to exclude pseudoachalasia 2

Step 3: High-Resolution Manometry (Gold Standard)

• High-resolution manometry (HRM) is essential for accurate diagnosis of esophageal spasm 1
• Modern diagnostic criteria require at least two premature contractions with distal latency <4.5 seconds in the context of normal lower esophageal sphincter relaxation 1, 5
• HRM has superior sensitivity to conventional manometry in recognizing atypical cases of achalasia and distal esophageal spasm 2
• Manometry serves to localize the lower esophageal sphincter for potential subsequent pH monitoring and evaluate peristaltic function 2

Step 4: Ambulatory pH Monitoring (If Diagnosis Unclear)

• Consider 24-hour ambulatory impedance-pH monitoring if GERD coexistence is suspected or diagnosis remains equivocal 1
• GERD frequently coexists with distal esophageal spasm and may contribute to symptom pathogenesis 5
• Withhold PPI therapy for 7 days before testing 2
• This definitively rules out GERD as a contributing factor 1

Additional Diagnostic Considerations

Rule Out Secondary Causes

• Query for infectious and inflammatory causes of secondary achalasia including recent COVID infections, Chagas disease risk, and eosinophilic disease 2
• Consider systemic sclerosis, mixed connective tissue disease, SLE, or Sjögren's disease as these can cause esophageal dysmotility 2
• In immunocompromised patients, consider infectious esophagitis (Candida, HSV, CMV) 2

Functional Luminal Impedance Planimetry (FLIP)

• FLIP can be a useful adjunct test particularly when diagnosis is equivocal on HRM 2
• Impaired esophagogastric junction opening (low distensibility index) can confirm outflow obstruction 2

Common Pitfalls to Avoid

• Do not assume esophageal origin without cardiac workup - this is the most critical error given mortality implications 1
• Do not rely on single diagnostic test - barium studies and manometry are complementary, and one may reveal abnormalities missed by the other 2, 3
• Do not skip biopsies even with normal-appearing mucosa - eosinophilic esophagitis can be latent and present in up to 9% of patients with dysphagia 2, 4
• Be aware that esophageal spasm is intermittent - it is almost impossible to completely rule out spasm given its episodic nature 3
• Consider that DES may progress to achalasia in some patients, requiring long-term follow-up 5

Initial Treatment Approach

First-Line Pharmacological Management

• Start with proton pump inhibitors (PPIs) especially when symptoms overlap with GERD 1
• Add smooth muscle relaxants (nitrates, calcium channel blockers) for symptom control 1, 5
• Consider neuromodulators (tricyclic antidepressants or SSRIs) for esophageal hypersensitivity and visceral analgesia 1
• Do NOT use metoclopramide - it is ineffective and potential harms outweigh benefits 1

Refractory Cases

• Per-oral endoscopic myotomy (POEM) is the preferred surgical approach for refractory distal esophageal spasm with success rates >90% 1
• Botulinum toxin injection may provide symptomatic benefit with good short and long-term results 6, 7
• Consider cognitive behavioral therapy, esophageal-directed hypnotherapy, and diaphragmatic breathing for associated hypervigilance 1